Idiopathic pulmonary fibrosis (IPF) is a type of lung disease that results in scarring (fibrosis) of the lungs for an unknown reason. Over time, the scarring gets worst and it becomes hard to take in a deep breath and the lungs cannot take in enough oxygen.
IPF is a form of interstitial lung disease, primarily involving the interstitium (the tissue and space around the air sacs of the lungs), and not directly affecting the airways or blood vessels. There are many other kinds of interstitial lung disease that can also cause inflammation and/or fibrosis, and these are treated differently. It is important to work with your doctor to determine if you have IPF or another form of interstitial lung disease.
The alveoli (air sacs) and the blood vessels within the lungs are responsible for delivering oxygen to the body, including the brain, heart, and other organs. All of the body’s functions depend upon delivery of a steady supply of oxygen. As lung tissue becomes scarred and thicker, it is more difficult for the lungs to transfer oxygen into the bloodstream. As a result, the brain, heart, and other organs do not get the oxygen they need to function properly.
In some cases, doctors can determine the cause of the fibrosis (scarring), but in many cases the cause remains unknown. When there is no known cause for the development of pulmonary fibrosis (and certain radiographic and/or pathologic criteria are met), the disease is called idiopathic pulmonary fibrosis or IPF.
Most people with IPF develop symptoms of cough and shortness of breath between the ages of 50 and 70 years. IPF is not common under the age of 50 years. Historically, more men have been diagnosed with IPF than women, but IPF in women appears to be on the rise. Occasionally, IPF occurs in members of the same family. When this happens, the disease is called Familial Pulmonary Fibrosis. The fact that PF runs in certain families has led many experts to believe that having certain genes (genetics) may be why some people get PF..
Causes
The term ‘idiopathic’ means the cause is not known. So it’s important to remember that no one is sure why you have developed this condition. We do know that IPF is more common in people who are smokers, or in people who have smoked in the past.
Environment – It is also more common if you have been exposed through your occupation to dust from wood, metal, textile or stone, or from cattle or farming.
Infection – Infection might be another cause. In some studies, IPF has been linked to certain viruses, including the Epstein Barr virus, which causes glandular fever. The herpes virus and hepatitis C have also been suggested as possible causes.
Acid Reflux – Doctors have noticed that some people with IPF also have a condition called gastro-oesophageal reflux disease (GORD). This is when the stomach contents leak back up into the oesophagus (gullet), causing the symptoms of heartburn. Reflux is very common in patients with any type of lung fibrosis, as the gullet becomes stretched by the fibrosis in the lung. Some experts think there could be a link between the two conditions.
Genetics (family history): Up to 20% of people with IPF have another family member with an interstitial lung disease. If more than one member of your family has IPF, the disease is called familial pulmonary fibrosis.
Symptoms
The two main symptoms of IPF are:
Breathlessness (also known as shortness of breath or dyspnea) – Usually the breathlessness of IPF first appears during exercise. Breathlessness can affect day-to-day activities such as showering, climbing stairs, getting dressed and eating. As scarring in the lungs gets worse, breathlessness may prevent all activities.
Chronic cough – About 85% of people with IPF have a chronic cough that has lasts longer than 8 weeks. This is often a dry cough, but some people may also cough up sputum or phlegm.
Other symptoms may include:
- Chest pain or tightness
- Unexplained weight loss
- Loss of appetite
- Tiredness and loss of energy
- Change of finger shape called “clubbing”
Treatment
Pulmonary rehabilitation can help you to cope with breathlessness and is an important part of treatment.
It includes –
- A physical exercise programme, designed individually for you; and
- Advice and information on keeping your lungs healthy and coping with breathlessness.
Sessions are held in hospitals, health centres and in places such as community halls. The groups are led by a qualified professional.
As well as providing support and reassurance, pulmonary rehabilitation can have a positive impact on your condition. It may help to increase the distance you can walk, help you feel less breathless and improve your sense of well-being.
Oxygen Therapy – Most patients with IPF require oxygen for getting about outside when their condition is more advanced. You might have an oxygen concentrator installed in your home. The concentrator takes normal air from the room and makes it richer in oxygen before you breathe it in. The machine is attached to tubing all around the house, so you are not confined to one room.
Medications – Steroid medication may be tried, usually in combination with immunosuppressant medicines. Previous studies showed that steroid medication improved symptoms in about 1 in 4 affected people. However, some doctors now wonder if the people for whom the steroids worked actually had another type of lung disease. If steroids are prescribed, a high dose is used at first and then reduced to a lower maintenance dose. If there is no improvement after a few months then the steroids are stopped. Some people who take steroids develop side-effects.
Prednisone, azathioprine (A-zah-THI-o-preen), and N-acetylcysteine (a-SEH-til-SIS-tee-in) have been used to treat IPF, either alone or in combination. However, experts have not found enough evidence to support their use.
A lung transplant may be an option. This is becoming more common, particularly in younger people who develop severe disease despite medication.
Alternative Treatment
Serrepeptase – This enzyme helps to thin mucous so it can be expelled from the lungs and the lymph system, reduce inflammation, and in the long term help reduce the actual fibrosis itself. Furthermore it will have some calming effect on the immune system that is attacking the lungs.
Colloidal Silver – This has many knock on effects throughout the body – (a) Metabolic energy and tissue oxygenation is improved, (b) cellular and liver detoxification is facilitated and (c) the T cell side of the Immune system is improved.
Liquorice – These are possibly the most effective supplement to calm down the overactive B cells so that the immune system stops attacking the lungs. It promotes healthy production of natural steroids from the Adrenal. These steroids are more effective than drug steroids and are in the form the body needs to help overcome inflammation, restore adrenal function and re-balance the immune system. Liquorice also has the property of being a good lung expectorant.
Hemp Oil – The correct balance of raw cold pressed essential fatty acids any health restoration programme will be limited in its scope, and they are known to be one aspect of resolving inflammation.
Organic Silicon – This form of silicon is by far the most effective in getting sufficient quantity to be absorbed into the body, possibly a six to 9 months course of this may be sufficient to build up the silica reserves of the body. Silica is important for repair, especially of collagen (this is involved in the lining of the lungs, expelling foreign particles/toxins, restoring elasticity to the collagen (e.g blood vessels, lining of the lungs etc) and facilitating lymph detoxification.
Organic Germanium – This helps to promote oxygenation of the tissues, and is a powerful aid to promoting cell detoxification and resolution of stubborn health disorders
Powerful pulmonary improving antioxidants, concentrated vitamin D3 in high dosages, N-Acetyl Cysteine, Alpha Lipoic Acid, Acetyl L-Carnitine and systemic enzymes are to name just a few.
Reference –
https://www.blf.org.uk/Page/Idiopathic-pulmonary-fibrosis-IPF
http://www.nhlbi.nih.gov/health/health-topics/topics/ipf/causes
http://www.lung.ca/lung-health/lung-disease/idiopathic-pulmonary-fibrosis/symptoms
https://www.blf.org.uk/Page/Causes-and-symptoms-of-IPF
http://www.nhs.uk/Conditions/pulmonary-fibrosis/Pages/Symptoms.aspx
http://www.modernmedicine.com/tag/idiopathic-pulmonary-fibrosis-resource-center-0
http://www.wsj.com/articles/new-treatments-for-deadly-idiopathic-pulmonary-fibrosis-1442251585